PRO score: predictive scoring system for visual outcomes after rhegmatogenous retinal detachment repair.

BACKGROUND/AIMS: To compare risk factors for poor visual outcomes in patients undergoing primary rhegmatogenous retinal detachment (RRD) repair and to develop a scoring system. METHODS: Analysis of the Primary Retinal detachment Outcomes (PRO) study, a multicentre interventional cohort of consecutive primary RRD surgeries performed in 2015. The main outcome measure was a poor visual outcome (Snellen VA ≤20/200). RESULTS: A total of 1178 cases were included. The mean preoperative and postoperative logMARs were 1.1±1.1 (20/250) and 0.5±0.7 (20/63), respectively. Multivariable logistic regression identified preoperative risk factors predictive of poor visual outcomes (≤20/200), including proliferative vitreoretinopathy (PVR) (OR 1.26; 95% CI 1.13 to 1.40), history of antivascular endothelial growth factor (VEGF) injections (1.38; 1.11 to 1.71), >1-week vision loss (1.17; 1.08 to 1.27), ocular comorbidities (1.18; 1.00 to 1.38), poor presenting VA (1.06 per initial logMAR unit; 1.02 to 1.10) and age >70 (1.13; 1.04 to 1.23). The data were split into training (75%) and validation (25%) and a scoring system was developed and validated. The risk for poor visual outcomes was 8% with a total score of 0, 17% with 1, 29% with 2, 47% with 3, and 71% with 4 or higher. CONCLUSIONS: Independent risk factors were compared for poor visual outcomes after RRD surgery, which included PVR, anti-VEGF injections, vision loss >1 week, ocular comorbidities, presenting VA and older age. The PRO score was developed to provide a scoring system that may be useful in clinical practice.

Survey of Vitreoretinal Specialists in the United States Regarding Telemedicine During the COVID-19 Pandemic.

Objective: To evaluate the attitudes, beliefs, and practice patterns of vitreoretinal specialists regarding the utilization of telemedicine during the COVID-19 pandemic, and to identify features which may predict future telemedicine use. Methods: An 11-question anonymous survey was completed electronically in July 2020 by vitreoretinal specialists practicing in the United States. Results: The survey response rate was 13.0% (361/2,774). Thirty-five respondents (9.7%) had used telemedicine before March 1, 2020; after March 1, 2020, 170 (47.1%) reported using telemedicine (p < 0.001). Of the 170 respondents who reported telemedicine use, a majority (65.3%;111/170) performed 0-5 patient visits per week. Female retina specialists, younger physicians, and those with prior telemedicine usage were more likely to use telemedicine. Barriers to telemedicine use included concern for misdiagnosis (332/361, 92.0%), inability to obtain optical coherence tomography imaging (330/361, 91.4%), inability to obtain fundus imaging (327/361, 90.6%), lack of access to and/or comfort with the technology (261/361, 72.3%), potential legal liability (229/361, 63.4%), and low reimbursement (227/361, 62.9%). The majority of respondents (225/361; 62.3%) reported that telemedicine without ancillary imaging was not an acceptable way to evaluate patients. However, 59.2% (214/361) would find telemedicine acceptable if remote imaging was available. Conclusions: The pandemic led to a rapid adoption of telemedicine by vitreoretinal specialists. The majority of specialists using telemedicine performed five or fewer visits per week. The availability of remote imaging may increase confidence in clinical outcomes with a subsequent increase in utilization of telemedicine by vitreoretinal specialists.

FACTORS AFFECTING RETINAL REDETACHMENT AFTER SILICONE OIL REMOVAL FOR RHEGMATOGENOUS RETINAL DETACHMENTS.

PURPOSE: To report factors affecting the retinal redetachment rate after silicone oil removal (SOR) following rhegmatogenous retinal detachment (RRD) repair. METHODS: Retrospective cohort study from December 1, 2014, to March 1, 2020, of 205 consecutive patients treated for RRD with silicone oil (SO) tamponade and subsequent SOR with at least 6-month follow-up. Primary outcome measure was the rate of retinal redetachment after SOR. RESULTS: The retinal redetachment rate after SOR was 18.5%. Preoperative macula and lens status, proliferative vitreoretinopathy, previous retinectomy, retinal detachment size, concomitant phacoemulsification, previous scleral buckling, and endolaser during SOR did not affect the redetachment rate after SOR. Previous SO exchange was associated with increased redetachment (OR 2.53, 95% CI [1.11-5.80], P = 0.0278). Twelve months of SO tamponade had lower redetachment rates compared with 3 months (OR 0.25, 95% CI [0.04-0.09], P = 0.048). Shorter SO tamponade (3 vs. 12 months) had better final visual outcomes after SOR (0.80 ± 0.61 vs. 1.41 ± 0.66, P < 0.0001). CONCLUSION: No preoperative or intraoperative factors in this analysis influenced the risk of redetachment after SOR except duration of SO tamponade and previous SO exchange. Although longer SO tamponade duration may be associated with lower rates of redetachment, visual outcomes may be worse.

CTNNB1 (ß-CATENIN) VITREORETINOPATHY: IMAGING CHARACTERISTICS AND SURGICAL MANAGEMENT.

PURPOSE: We report a patient with CTNNB1-associated vitreoretinopathy. We discuss imaging findings and surgical management. METHODS: Case report. RESULTS: An 18-month-old girl with microcephaly, failure to thrive, developmental delay, and chronic rhinitis presented with bilateral central and peripheral tractional retinal detachments and an anomalous retinal vasculature. She underwent multimodal imaging and genetic testing, and we discuss successful surgical management. CONCLUSION: CTNNB1 mutations can cause a vision-threatening vitreoretinopathy. We recommend CTNNB1 to be considered as part of the workup of patients presenting with familial exudative vitreoretinopathy-like clinical findings, especially if there are systemic manifestations.

Scleral Buckling for Primary Retinal Detachment: Outcomes of Scleral Tunnels versus Scleral Sutures.

PURPOSE: There are primarily two techniques for affixing the scleral buckle (SB) to the sclera in the repair of rhegmatogenous retinal detachment (RRD): scleral tunnels or scleral sutures. METHODS: This retrospective study examined all patients with primary RRD who were treated with primary SB or SB combined with vitrectomy from January 1, 2015 through December 31, 2015 across six sites. Two cohorts were examined: SB affixed using scleral sutures versus scleral tunnels. Pre- and postoperative variables were evaluated including visual acuity, anatomic success, and postoperative strabismus. RESULTS: The mean preoperative logMAR VA for the belt loop cohort was 1.05 ± 1.06 (Snellen 20/224) and for the scleral suture cohort was 1.03 ± 1.04 (Snellen 20/214, p = 0.846). The respective mean postoperative logMAR VAs were 0.45 ± 0.55 (Snellen 20/56) and 0.46 ± 0.59 (Snellen 20/58, p = 0.574). The single surgery success rate for the tunnel cohort was 87.3% versus 88.6% for the suture cohort (p = 0.601). Three patients (1.0%) in the scleral tunnel cohort developed postoperative strabismus, but only one patient (0.1%) in the suture cohort (p = 0.04, multivariate p = 0.76). All cases of strabismus occurred in eyes that underwent SB combined with PPV (p = 0.02). There were no differences in vision, anatomic success, or strabismus between scleral tunnels versus scleral sutures in eyes that underwent primary SB. CONCLUSION: Scleral tunnels and scleral sutures had similar postoperative outcomes. Combined PPV/SB in eyes with scleral tunnels might be a risk for strabismus post retinal detachment surgery.

Central retinal vein occlusion after laparoscopic sleeve gastrectomy in an 18-year-Old female.

PURPOSE: To describe a case of central retinal vein occlusion (CRVO) in a young adult found to have elevated homocysteine after bariatric surgery and to review risk factors for CRVO in younger patients. OBSERVATIONS: An 18-year-old female presented with a CRVO and severe cystoid macular edema (CME). She was normotensive, not on medications, and without known heritable hypercoagulable disease. Her medical history was notable for bariatric surgery and subsequently she was found to have nutritional deficiency, anemia, and elevated homocysteine. Her elevated homocysteine may have induced a hypercoaguable state that predisposed her to developing a CRVO. Treatment with bevacizumab dramatically improved her macular edema and visual acuity. Nutritional supplementation was initiated. CONCLUSIONS: Elevated homocysteine in the setting of nutritional deficiency from prior bariatric surgery may cause a hypercoaguable state and is a potential risk factor for CRVO. IMPORTANCE: With the increasing prevalence of obesity and bariatric surgery, it is important to consider associated nutrient deficiency as a potential cause of induced hypercoagulability and increased risk for CRVO. Identification of these at-risk patients is especially important given this condition is easily treatable and may be present in younger individuals. To the best of our knowledge, this is the first reported case of a CRVO with elevated homocysteine in the setting of nutrient deficiencies from laparoscopic sleeve gastrectomy.

Fluctuations in Central Subfield Thickness Associated With Worse Visual Outcomes in Patients With Diabetic Macular Edema in Clinical Trial Setting.

PURPOSE: This study examines the relationship between fluctuations in central subfield thickness (CST) and visual acuity (VA) in patients with diabetic macular edema (DME) using data from 2 large clinical trials. DESIGN: Clinical cohort study using post hoc analysis of clinical trial databases. METHODS: Standard deviation (SD) of all recorded CSTs for each patient during the study period were used to quantify the fluctuations in CST. Patients from each protocol were grouped into quartiles based on the CST SD. Eyes with at least 3 CSTs and VA at 1 year were included. The main outcome measures were VA at 1 and 2 years for each protocol, stratified by SD quartile. RESULTS: A total of 1197 eyes were included in the analysis. There were significant VA differences based on CST SD quartile for both protocols while adjusting for mean baseline VA, baseline CST, lens status, hemoglobin A1c, and treatment arm. At week 52 in protocol T, the difference between the first and fourth quartiles was -1.61 Early Treatment Diabetic Retinopathy Study letters (95% confidence interval [CI] -3.52 to 0.30, P = .0986). At week 104, this difference was -3.59 letters (95% CI -6.17 to -1.00, P = .0066). In protocol V, at week 52, the difference between the first and fourth quartiles was -3.04 letters (95% CI -4.18 to -1.91, P < .0001). At week 104, this difference was -2.35 letters (95% CI -3.58 to -1.13, P = .0005). CONCLUSION: Large fluctuations in CST may portend worse VA outcomes at the 2-year end point in patients with DME.

Comparative Incidence of Postoperative Hemorrhage in Vitreoretinal Surgery in Patients on Anti-Coagulants.

BACKGROUND AND OBJECTIVE: Data regarding hemorrhagic complications of direct oral anticoagulants (DOACs) and vitreoretinal surgery are limited. PATIENTS AND METHODS: Multicenter analysis of longitudinal, aggregated electronic health records of patients undergoing pars plana vitrectomy (PPV) with no prior history of ocular hemorrhage. Retrospective analysis of patients undergoing PPV between January 1, 2013, and December 31, 2019. The main outcomes were development of postoperative hemorrhagic complications within 1 month following vitreoretinal surgery. RESULTS: A total of 58,131 eyes underwent PPV, with 2,956 (5.1%) on anticoagulant medication prior to surgery. Eight hundred twenty-eight eyes (1.4%) developed a postoperative hemorrhage. Of eyes with anticoagulation use, 50 of 2,956 (1.29%) developed a hemorrhage, whereas 778 of 55,175 (1.41%) of the eyes with no prior anticoagulation use developed a postoperative hemorrhage (P = .2107). CONCLUSION: Use of DOACs prior to vitreoretinal surgery does not appear to be associated with increased rates of postoperative intraocular hemorrhage. [Ophthalmic Surg Lasers Imaging Retina. 2021;52:374-379.].

CLINICAL UTILITY OF BETA-D-GLUCAN TESTING FOR ENDOGENOUS FUNGAL CHORIORETINITIS OR ENDOPHTHALMITIS.

PURPOSE: To evaluate serum beta-D-glucan (BDG) as a biomarker for endogenous fungal eye infection. METHODS: Retrospective case-control study of 88 patients with a BDG test and eye examination at UPenn (2013-2018). Cases had endogenous fungal chorioretinitis or endophthalmitis diagnosed by eye examination and confirmed with positive culture; controls were without these fungal eye findings. Charts were reviewed for BDG values, blood/vitreous cultures, and eye examinations. Outcomes were BDG sensitivity, specificity, positive predictive value, and negative predictive value for fungal chorioretinitis or endophthalmitis, using prespecified BDG cut-off points of ≥80, ≥250, and ≥500 pg/mL as test positive. RESULTS: Cases included six chorioretinitis and four endophthalmitis patients. Controls included 78 patients without chorioretinitis or endophthalmitis. Defining BDG ≥80 pg/mL as test positive, the BDG sensitivity (95% confidence interval) was 66.7% (22.3%-95.7%) for chorioretinitis and 100% (39.8%-100%) for endophthalmitis. The specificity was 74.4% (63.2%-83.6%) when BDG values ≥80 pg/mL were test positive, and 85.9% (76.2%-92.7%) when values ≥250 pg/mL were test positive. For a 1% endophthalmitis prevalence and BDG cut-off value of ≥80 pg/mL, the positive predictive value was 3.8% (2.4%-5.2%) and negative predictive value was 100% (99.1%-100%). CONCLUSION: For endogenous fungal endophthalmitis, BDG's sensitivity and specificity seem good and the negative predictive value is high; a larger ophthalmic study is indicated.

ENHANCED S-CONE SYNDROME: VISUAL FUNCTION, CROSS-SECTIONAL IMAGING, AND CELLULAR STRUCTURE WITH ADAPTIVE OPTICS OPHTHALMOSCOPY.

PURPOSE: To describe in detail the phenotype of a patient with enhanced S-cone syndrome. METHODS: We describe a 13-year-old boy who presented with blurred vision, vitreous cells, cystoid macular edema refractory to steroid treatment, and a negative uveitic workup. The patient underwent a complete ophthalmic examination, full-field electroretinograms (ffERG), automatic static perimetry and multimodal imaging with spectral domain optical coherence tomography, and adaptive optics scanning laser ophthalmoscopy (AOSLO). RESULTS: Spectral domain optical coherence tomography demonstrated cystoid macular edema and a hyperthick, delaminated midperipheral retina. Fluorescein angiography did not demonstrate macular leakage. Rod-mediated ffERGs were undetectable, and there was a supernormal response to short-wavelength stimuli compared with photopically matched longer wavelengths of light consistent with enhanced S-cone syndrome. Gene screening was positive for compound heterozygous mutations NR2E3: a known (c.119-2 A>C) and a novel (c.119-1G>A) mutation. By perimetry, sensitivities were normal or above normal for short-wavelength stimuli; there was no detectable rod-mediated vision. AOSLO demonstrated higher than normal cone densities in the perifoveal retina and evidence for smaller outer segment cone diameters. CONCLUSION: Evidence for supernumerary cones (at least twice the normal complement) by AOSLO and spectral domain optical coherence tomography was associated with supernormal S-cone sensitivities and electroretinogram responses confirming previous in vivo findings in postmortem human specimens. Smaller than normal cones in enhanced S-cone syndrome may represent "hybrid" photoreceptors analogous to the rd7/rd7 murine model of the disease.

Cryptococcal Meningitis Presenting as Endophthalmitis in an Otherwise Asymptomatic Patient.

Purpose: Cryptococcus neoformans is a budding yeast that has a tropism for the central nervous system where it may cause meningitis, which has a high mortality rate. Endophthalmitis is rare and typically occurs in the setting of meningitis and disseminated disease. This report describes an atypical presentation of cryptococcal endophthalmitis and outlines the appropriate workup and management for this disease. Methods: A case report is presented. Results: A 66-year-old man on chemotherapy who presented with blurry vision without any extraocular symptoms was diagnosed with cryptococcal endophthalmitis and found to have underlying bloodstream and central nervous system infection on subsequent workup. He was treated with intravenous and intravitreal antifungals and remained systemically well, although a large subretinal mass lesion remained stable at 2-month follow-up. Conclusions: Cryptococcal endophthalmitis may be the initial presentation of disseminated cryptococcal disease. If vitreous cultures grow Cryptococcus, clinicians should presume that meningitis is present, regardless of symptoms, until proven otherwise.

Clinical Presentation of Rhegmatogenous Retinal Detachment during the COVID-19 Pandemic: A Historical Cohort Study.

PURPOSE: To investigate the effect of the coronavirus disease 2019 (COVID-19) pandemic on the clinical presentation of acute, primary rhegmatogenous retinal detachment (RRD). DESIGN: Single-center, consecutive case series with historical controls. PARTICIPANTS: Consecutive patients seeking treatment for primary RRD in a 50-day period during the COVID-19 pandemic (March 9-April 27, 2020) and the corresponding 50-day period during the previous year (March 4-April 22, 2019) in the United States. METHODS: The cohorts were compared to assess demographic variables and clinical presentations. Multivariate logistic regression was used to identify factors predictive of presenting macular attachment status. MAIN OUTCOME MEASURES: The primary outcome was the proportion of patients with macula-on RRD at presentation. Secondary outcomes included visual acuity (VA), duration of symptoms before presentation, proportion seeking treatment within 1 day of symptom onset, and presence of primary proliferative vitreoretinopathy (PVR). RESULTS: Eighty-two patients were included in the 2020 cohort compared with 111 patients in the 2019 primary control cohort. Demographic factors were similar between the groups. Significantly fewer patients demonstrated macula-on RRD in the 2020 cohort (20/82 patients [24.4%]) than in the 2019 cohort (55/111 patients [49.5%]; P = 0.001). Patients in the 2020 cohort showed worse median VA at presentation (1.00 logarithm of the minimum angle of resolution [logMAR; Snellen equivalent, 20/200] in 2020 vs. 0.48 logMAR [Snellen equivalent, 20/60] in 2019; P = 0.008), fewer patients sought treatment within 1 day of symptoms (16/80 patients [19.5%] in 2020 vs. 41/106 patients [36.9%] in 2019; P = 0.005), and a greater proportion demonstrated primary PVR (11/82 patients [13.4%] in 2020 vs. 5/111 patients [4.5%] in 2019; P = 0.03). In multivariate analysis, younger age (P = 0.03) and established patient status (P = 0.02) were independent predictors of macula-on status in the 2020 cohort. CONCLUSIONS: Patients with primary RRD during the 2020 COVID-19 pandemic were less likely to have macula-on disease and more likely to delay seeking treatment and to show worse vision and PVR.

Practice Patterns and Responsiveness to Simulated Common Ocular Complaints Among US Ophthalmology Centers During the COVID-19 Pandemic.

IMPORTANCE: The coronavirus disease 2019 (COVID-19) pandemic has drastically changed how comprehensive ophthalmology practices care for patients. OBJECTIVE: To report practice patterns for common ocular complaints during the initial stage of the COVID-19 pandemic among comprehensive ophthalmology practices in the US. DESIGN, SETTING, AND PARTICIPANTS: In this cross-sectional study, 40 private practices and 20 university centers were randomly selected from 4 regions across the US. Data were collected on April 29 and 30, 2020. INTERVENTIONS: Investigators placed telephone calls to each ophthalmology practice office. Responses to 3 clinical scenarios-refraction request, cataract evaluation, and symptoms of a posterior vitreous detachment-were compared regionally and between private and university centers. MAIN OUTCOMES AND MEASURES: The primary measure was time to next appointment for each of the 3 scenarios. Secondary measures included use of telemedicine and advertisement of COVID-19 precautions. RESULTS: Of the 40 private practices, 2 (5%) were closed, 24 (60%) were only seeing urgent patients, and 14 (35%) remained open to all patients. Of the 20 university centers, 2 (10%) were closed, 17 (85%) were only seeing urgent patients, and 1 (5%) remained open to all patients. There were no differences for any telemedicine metric. University centers were more likely than private practices to mention preparations to limit the spread of COVID-19 (17 of 20 [85%] vs 14 of 40 [35%]; mean difference, 0.41; 95% CI, 0.26-0.65; P < .001). Private practices had a faster next available appointment for cataract evaluations than university centers, with a mean (SD) time to visit of 22.1 (27.0) days vs 75.5 (46.1) days (mean difference, 53.4; 95% CI, 23.1-83.7; P < .001). Private practices were also more likely than university centers to be available to see patients with flashes and floaters (30 of 40 [75%] vs 8 of 20 [40%]; mean difference, 0.42; 95% CI, 0.22-0.79; P = .01). CONCLUSIONS AND RELEVANCE: In this cross-sectional study of investigator telephone calls to ophthalmology practice offices, there were uniform recommendations for the 3 routine ophthalmic complaints. Private practices had shorter times to next available appointment for cataract extraction and were more likely to evaluate posterior vitreous detachment symptoms. As there has not been a study examining these practice patterns before the COVID-19 pandemic, the relevance of these findings on public health is yet to be determined.

Scleral fixated secondary intraocular lenses: a review of recent literature.

PURPOSE OF REVIEW: A variety of techniques exist for secondary intraocular lens (IOL) implantation. Of note, scleral fixated intraocular lenses have become more popular with a variety of techniques, both with and without use of sutures. Herein, we focus on reviewing recently published studies describing the long-term outcomes of scleral fixation techniques. RECENT FINDINGS: Although initial papers describing novel techniques often report short-term outcomes, several studies have recently described intermediate and long-term outcomes for scleral fixated IOLs, albeit all being retrospective case series. Suture fixation methods with long-term follow-up, up to a minimum of 50 months, report dislocation rates between 0 and 15%. Sutureless scleral fixation techniques have increased in popularity the past several years. Although they appear to have a lower rate of IOL dislocation: several studies have reported 0% and one study 8%. The follow-up period for sutureless scleral fixation technique studies, however, is shorter with most studies reporting follow-up of less than a year. Rates of retinal detachment vary between individual studies, but are similar for both suture fixation and sutureless with the majority of studies reporting a rate between 0 and 5%. These studies show that long-term outcomes are important considerations in surgical decision-making. SUMMARY: Scleral fixation techniques have shown long-term durability and safety in recent retrospective studies. Comparison of techniques has been limited, and more robust studies may be required to provide stronger anatomic, functional, and comparative data.

Cytomegalovirus retinitis in an immunocompetent host after complicated cataract surgery.

PURPOSE: To report a case of an immunocompetent patient who developed cytomegalovirus (CMV) retinitis after complicated cataract surgery resulting in aphakia. OBSERVATIONS: A 67-year-old man with type 2 diabetes developed retinitis six months after cataract surgery that resulted in aphakia. Anterior chamber DNA testing was positive for CMV. Comprehensive systemic work-up revealed no immune insufficiency. The retinitis was successfully treated with intravitreal foscarnet and extended oral valgancyclovir treatment, however, he subsequently developed rhegmatogenous retinal detachment. CONCLUSION AND IMPORTANCE: CMV retinitis may occur in immunocompetent patients in the setting of aphakia and prolonged topical steroid use.

Age-related macular degeneration therapy: a review.

PURPOSE OF REVIEW: The purpose of this review is to describe the current clinical landscape of potential future therapies for both nonexudative (dry) and exudative (wet) age-related macular degeneration (AMD). We highlight some of the more promising treatments that are furthest along in development. RECENT FINDINGS: Patients with dry AMD have long been hoping for a highly efficacious treatment that may slow disease progression or even help regain vision. Patients with wet AMD have many effective treatment options but still there are those who have suboptimal responses or are burdened by the high frequency of treatment. We detail exciting new concepts and targets for novel medications. Specifically, for dry AMD we discuss research looking at complement inhibition, neuroprotection, visual cycle modulators, cell-based therapies, and anti-inflammatory agents. For wet AMD we summarize new, potentially more durable anti-vascular endothelial growth factor agents, extended release options, and gene therapy. SUMMARY: There are promising new strategies for AMD. Many of the potential new treatments are in or have recently completed phase 2 or phase 3 clinical trials with promising results thus far, including some that have received US Food and Drug Administration approval. Additional therapeutic breakthroughs will likely continue to occur thanks to the number of clinical trials that are nearing the finish line.

RECURRENT BRANCH RETINAL ARTERY OCCLUSION FROM SUSAC SYNDROME: CASE REPORT AND REVIEW OF LITERATURE.

PURPOSE: We report a patient with branch retinal artery occlusion predominant Susac syndrome and review the literature on current diagnostic modalities and therapeutic approaches for this rare condition. METHODS: Case report. RESULTS: A 23-year-old white male presented with a 5-month history of a scotoma in his right eye vision, headaches, and intermittent paresthesias of the lower extremities. Ophthalmic examination was normal with the exception of a hypopigmented small retinal area inferonasal to the fovea in the right eye. Optical coherence tomography demonstrated inner retinal atrophy and optical coherence tomography angiography showed deep capillary layer vessel dropout in both eyes. Ultra-wide-field fluorescein angiography revealed bilateral arteriolar wall hyperfluorescence. Corpus callosal lesions were present on magnetic resonance imaging. Lumbar puncture demonstrated elevated protein. Audiogram was normal. A diagnosis of Susac syndrome was made. The patient initially received oral steroids followed by intravenous steroids and mycophenolate mofetil because of new branch retinal artery occlusions. Thirteen months after initial presentation, the vision was stable at 20/20 in both eyes. CONCLUSION: It is a diagnostic challenge when recurrent branch retinal artery occlusion secondary to Susac syndrome presents without the classic symptoms. Optical coherence tomography angiography and subtle magnetic resonance imaging findings aided in early recognition of the diagnosis in our patient. Ultra-wide-field fluorescein angiography was an important tool in disease monitoring. Aggressive management with intravenous corticosteroids and initiation of immunosuppression resulted in long-term preservation of vision and improvement in visual field.

Safety of Same-Eye Subretinal Sequential Readministration of AAV2-hRPE65v2 in Non-human Primates.

We have demonstrated safe and effective subretinal readministration of recombinant adeno-associated virus serotype (rAAV) to the contralateral eye in large animals and humans even in the setting of preexisting neutralizing antibodies (NAbs). Readministration of AAV to the same retina may be desirable in order to treat additional areas of the retina not targeted initially or to boost transgene expression levels at a later time point. To better understand the immune and structural consequences of subretinal rAAV readministration to the same eye, we administered bilateral subretinal injections of rAAV2-hRPE65v2 to three unaffected non-human primates (NHPs) and repeated the injections in those same eyes 2 months later. Ophthalmic exams and retinal imaging were performed after the first and second injections. Peripheral blood monocytes, serum, and intraocular fluids were collected at baseline and post-injection time points to characterize the cellular and humoral immune responses. Histopathologic and immunohistochemical studies were carried out on the treated retinas. Ipsilateral readministration of AAV2-hRPE65v2 in NHPs did not threaten the ocular or systemic health through the time span of the study. The repeat injections were immunologically and structurally well tolerated, even in the setting of preexisting serum NAbs. Localized structural abnormalities confined to the outer retina and retinal pigmented epithelium (RPE) after readministration of the treatment do not differ from those observed after single or contralateral administration of an AAV carrying a non-therapeutic transgene in NHPs and were not observed in a patient treated with the nearly identical, FDA-approved, AAV2-hRPE65v2 vector (voretigene neparvovec-rzyl), suggesting NHP-specific abnormalities.